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      Tethered cord: natural history, surgical outcome and risk for Chiari malformation 1 (CM1) : A review of 110 detethering

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          Abstract

          The surgical results of this series of occult spina bifida seem better than the natural history registered in the long pre-operative period in terms of neurological deterioration. The major contribution to this result is attributed to neurophysiological monitoring that lowers the risks of permanent damage and increases the percentage of effective detethering. The present series of TCS, due to conus and filar lipoma, documents that CM1 is a really rare association occurring in less than 6% of the patients, despite the low position of conus. The detethering procedure did not influence the tonsillar position, thus excluding the correlation between the tethering and the tonsillar descent. The genetic alteration documented in a girl reinforces the hypothesis of a rare complex polymaformative picture deserving multiple procedures according to the prevailing clinical symptoms.

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          Association of Chiari malformation type I and tethered cord syndrome: preliminary results of sectioning filum terminale.

          Thomas Milhorat, Paolo Bolognese, Misao Nishikawa … (2009)
          The pathogenesis of CM-I is incompletely understood. We describe an association of CM-I and TCS that occurs in a subset of patients with normal size of the PCF. The prevalence of TCS was determined in a consecutively accrued cohort of 2987 patients with CM-I and 289 patients with low-lying cerebellar tonsils (LLCT). Findings in 74 children and 244 adults undergoing SFT were reviewed retrospectively. Posterior cranial fossa size and volume were measured using reconstructed 2D computed tomographic scans and MR images. Results were compared to those in 155 age- and sex-matched healthy control individuals and 280 patients with generic CM-I. The relationships of neural and osseus structures at the CCJ and TLJ were investigated morphometrically on MR images. Intraoperative CDU was used to measure anatomical structures and CSF flow in the lumbar theca. Tethered cord syndrome was present in 408 patients with CM-I (14%) and 182 patients with LLCT (63%). In 318 patients undergoing SFT, there were no significant differences in the size or volume of the PCF as compared to healthy control individuals. Morphometric measurements demonstrated elongation of the brain stem (mean, 8.3 mm; P < .001), downward displacement of the medulla (mean, 4.6 mm; P < .001), and normal position of the CMD except in very young patients. Compared to patients with generic CM-I, the FM was significantly enlarged (P < .001). The FT was typically thin and taut (mean transverse diameter, 0.8 mm). After SFT, the cut ends of the FT distracted widely (mean, 41.7 mm) and CSF flow in the lumbar theca increased from a mean of 0.7 cm/s to a mean of 3.7 cm/s (P < .001). Symptoms were improved or resolved in 69 children (93%) and 203 adults (83%) and unchanged in 5 children (7%) and 39 adults (16%) and, worse, in 2 adults (1%) over a follow-up period of 6 to 27 months (mean, 16.1 months +/- 4.6 SD). Magnetic resonance imaging 1 to 18 months after surgery (mean, 5.7 months +/- 3.8 SD) revealed upward migration of the CMD (mean, 5.1 mm, P < .001), ascent of the cerebellar tonsils (mean, 3.8 mm, P < .001), reduction of brain stem length (mean, 3.9 mm, P < .001), and improvement of scoliosis or syringomyelia in some cases. Chiari malformation type I/TCS appears to be a unique clinical entity that occurs as a continuum with LLCT/TCS and is distinguished from generic CM-I by enlargement of the FM and the absence of a small PCF. Distinctive features include elongation and downward displacement of the hindbrain, normal position of the CMD, tight FT, and reduced CSF flow in the lumbar theca. There is preliminary evidence that SFT can reverse moderate degrees of tonsillar ectopia and is appropriate treatment for cerebellar ptosis after Chiari surgery in this cohort.
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            Tethered cord syndrome and the conus in a normal position.

            Daryl E Warder, Patrick W. Oakes (1993)
            The intent of this article is to propose the existence of the tethered cord syndrome in patients whose conus is in a normal position. The tethered cord syndrome has been a well-recognized entity, occurring in the pediatric and adult population. A central tenet to this syndrome is that the conus medullaris must be "abnormally" low, regardless of the tethering lesion. Our 12-year series of 73 patients with tethered cord syndrome included 13 patients in whom the cord terminated at or above the L1-L2 space. These patients otherwise displayed characteristics usually associated with the patient with an abnormally low conus. Most patients with progressive neurological deterioration responded to surgery. The preoperative radiographic evaluation and the operative findings usually demonstrated a thickened filum. Tethered cord syndrome may occur in the patient who has a conus in the "normal" position.
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              Results of the section of the filum terminale in 20 patients with syringomyelia, scoliosis and Chiari malformation.

              R Doménech, J Llenas, José Royo … (2005)
              Spinal cord traction caused by a tight filum terminale may be considered a pathogenic mechanism involved in the development of syringomyelia, the Chiari malformation (type I) and scoliosis. Section of the filum terminale is proposed as a useful surgical approach in these conditions. Between April 1993 and July 2003, a total of 20 patients (8 men and 12 women) with a mean age of 33.5 years underwent section of the filum terminale with or without opening of the dural sac through a standard sacrectomy. Eight patients suffered from scoliosis, 5 from syringomyelia, 2 from Chiari malformation and 5 with a combination of these conditions. After section of the filum terminale, patients with syringomyelia showed an early clinical improvement of dysaesthesia, thermo-anaesthesia, hypo-aesthesia and walking difficulties. Rising of the medullary conus was also observed. In patients with scoliosis, back pain improved dramatically and a curve reduction was noticed, although progression of the curve was observed in one case. In patients with Chiari malformation, headache, dysaesthesia and paraparesis disappeared. Section of the filum terminale is a useful strategy in the treatment of scoliosis, syringomyelia and the Chiari malformation, and offers a new aetiological basis for the understanding of these three disorders.
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                Author and article information

                Contributors
                : +39-02-2394421 , +39-02-70635017 , lvalentini@istituto-besta.it
                Journal
                Journal ID (nlm-ta): Neurol Sci
                Title: Neurological Sciences
                Publisher: Springer Milan (Milan )
                ISSN (Print): 1590-1874
                ISSN (Electronic): 1590-3478
                Publication date (Electronic): 16 September 2011
                Publication date PMC-release: 16 September 2011
                Publication date (Print): December 2011
                Volume: 32
                Issue: Suppl 3
                Pages: 353-356
                Affiliations
                [1 ]Department of Neurosurgery, Fondazione Istituto Neurologico “Carlo Besta”, Via Celoria 11, 20133 Milan, Italy
                [2 ]Services of Neuroradiology, Fondazione Istituto Neurologico “Carlo Besta”, Milan, Italy
                [3 ]Services of Neurophysiology, Fondazione Istituto Neurologico “Carlo Besta”, Milan, Italy
                [4 ]Department of Pediatric Surgery, Ospedale Pediatrico “Vittore Buzzi”, Milan, Italy
                Article
                Publisher ID: 745
                DOI: 10.1007/s10072-011-0745-7
                PMC ID: 3249026
                PubMed ID: 21922314
                SO-VID: 15b1a1e4-03ef-4475-8df6-5d0ad8d6ff13
                Copyright © © The Author(s) 2011
                History
                Categories
                Subject: Associated Malformations
                Custom metadata
                issue-copyright-statement © Springer-Verlag 2011

                ScienceOpen disciplines: Neurosciences
                Keywords: tethered cord,detethering,chiari 1 malformation,children
                Data availability:
                ScienceOpen disciplines: Neurosciences
                Keywords: tethered cord, detethering, chiari 1 malformation, children

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